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Interviewer: You've been diagnosed with a sarcoma. What now? We'll talk about that next on The Scope.

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Interviewer: Dr. Lor Randall is the Director of Sarcoma Services at Huntsman Cancer institute. Let's talk about sarcoma which is a particularly nasty cancer. It's not a diagnosis I'd imagine you enjoy giving to a patient, but first of all, a little background. What is sarcoma?

Dr. Randall: Well first off, sarcoma in general, is a group of tumors. It's over a 100 different diagnoses, and in the adult patient population, so we're talking about patients in their 30s, 40s, and 50s, and beyond, it only makes up about 1% of cancers, fortunately. Unfortunately, in the pediatric population, so those younger people, it makes up about 15% of cancers.

And by definition, a sarcoma arises from what we call connective tissue. Connective tissue is sort of the glue that holds the body together. You have your organs. You've got your prostate. You've got your breasts. You've got your colon. You have all of those organs that can lead to cancers. which we generally call carcinomas, and then those organs are suspended in connective tissue, which also includes muscles and bones and ligaments. The sarcomas arise from those types of tissues.

Interviewer: And I've heard it defined as more of a bone cancer. Is that accurate? Does it affect people's bones more often than those other connective tissues?

Dr. Randall: Bone is one of the more common forms. In the adults, we'll see something called chondrosarcoma arising in bone. In children we'll see osteosarcoma and another entity called Ewing's sarcoma. But there are unfortunately plenty of soft tissue sarcomas as well.

Interviewer: And what causes a sarcoma?

Dr. Randall: Well, we break up sarcomas into two classes, those with a what we call distinct molecular signature or translocation where there is a molecular event that we know is oncogenic or cancer-forming versus those that are more haphazard. Bad luck, if you will. The cell goes awry. It doesn't do what it's supposed to do, and cancer, or sarcoma in this case, arises from it.

Interviewer: That second way is the way that most other types of cancer happen. Can you explain more about the first cause?

Dr. Randall: Sure. There are definitely, not just exclusive to sarcomas, there are definitely cancers are forms of blood cancers. There are forms of a variety of other colon cancers where there is a known molecular defect that drives the formation of the cancer.

Interviewer: I look into somebody's genome and I can see that you have the propensity for that. Is that what we're talking about?

Dr. Randall: Well, that's very interesting you bring that up. I'm not specifically talking about necessarily predisposition. I'm talking about the actual event itself. So if I were to have a Ewing's sarcoma, my sarcoma would have this particular EWS/FLY1 translocation, but that doesn't mean that that EWS/FLY1 translocation, that molecular marker we're referring to, is present in all my cells.

But we actually have investigators here at Huntsman Cancer Institute and Primary Children's Hospital looking at the predisposition phenomenon you're referring to, that there may be some sort of genetic underlying predisposition that enables that translocation to then arise and form that specific tumor.

Interviewer: You had mentioned that diagnosing a sarcoma is a complex process, the complexities of sarcoma diagnosis. Can you elaborate on that? What do you mean by that?

Dr. Randall: Well, lots of people get lumps and bumps throughout their life, and fortunately most of those lumps and bumps are not sarcomas. But when they are, sometimes they're not actually worked up. They are seen by a very well intended provider who thinks it's a lipoma for example. And they take it out which we call an oops procedure, for unplanned surgery meaning that they've actually taken this thing out when what should have happened is a biopsy, meaning you take a sample of it.

You take that sample. You figure out what you're dealing with. You take it before an experienced tumor board with surgeons, medical oncologists, radiation therapists, and a variety of other practitioners, and you come up with a specific treatment plan. Unfortunately, that does not always happen, and then you sometimes have a compromised situation.

Interviewer: So it sounds like we've just learned one lesson here, that if I have any sort of lump or bump that a physician says, "That's cancer, let's just take it out." I should insist that it be analyzed more closely.

Dr. Randall: Right. We're talking about a concept that makes sense, but we have to be careful here. I mean, every single lump and bump is definitely not a sarcoma, so we have to be discretionary. But an experienced physician or allied health care provider needs to consult with experts when there's anything suspicious, such as it's growing, it's behaving in a way that isn't typical of a lipoma, which is that it's non-tender, and it's just sitting there, and it's not increasing in size.

Interviewer: And the treatments for sarcomas aren't quite as elegant as maybe some other cancer treatments I understand. Because it is a connective tissue treatment, sometimes it happens in the bones, it means the limb has to be removed.

Dr. Randall: Yeah. And I would affectionately say that the treatments are very elegant.

Interviewer: Oh, okay. Well sure. But, I mean, a limb removal. That must be pretty shocking for a patient. Is that kind of the only option if somebody has a bone sarcoma?

Dr. Randall: No. Not at all.

Interviewer: Okay, good.

Dr. Randall: The vast majority of patients actually undergo something called limb salvage. We don't do many amputations at all. Sometimes, if, for example, if a sarcoma arises about the ankle, the options for a good functional outcome as well as a cancer cure are a what we call below-knee amputation, but anywhere above that we almost invariably are able to save the limb.

Interviewer: Oh, okay.

Dr. Randall: And give them good functionality.

Interviewer: You describe sarcoma as a horrific type of cancer. I'm not used to physicians using such pointed language. Normally it's difficult to get that type of language out of a physician, but yet there those words are. Explain more about that.

Dr. Randall: Well, people often comment my passions may get the better of me, but it is . . . Many of the patients undergo not only a life-threatening or experience a life-threatening situation, but a functionally, potentially functionally devastating problem. Not only do they have to face the fear of dying or their loved one fear of dying, but they also have to face the fact that their life from a functional standpoint is going to be dramatically altered. And that's, you know, insult to injury.

Interviewer: What do you say to a patient? I mean, for somebody that's listening because they or a loved one has just been diagnosed with this type of cancer, to hear that would be very discouraging I think. How do you work through that process with patients?

Dr. Randall: Well, it's very nuanced discussion. Not only do you have to talk about the treatments that affect their being cured of their cancer, but then you also have to consider, what are their lifestyle demands? Because many of these sarcomas, and we're making broad strokes here, but many of these sarcomas do, as we talked about, compromise functionality.

And so, if it's a young person who is growing, still has growth in front of them, with a bone sarcoma options are different than someone who is maybe in their 50s or 60s where they're looking to have 20 to 30 good years as opposed to the other person. You have to have a lot of discussions about what their expectations are as a family or as an individual with what their life is going to look like not only being cured but from a functional standpoint in society.

Interviewer: And since, unlike a lot of cancers which we know one of the risk factors is just getting older, this one does affect a lot of children as you mentioned. What are some of the challenges of treating children with sarcoma?

Dr. Randall: Well, I mean, that's one of the horrors, right? As a parent I can tell you, I'm far more worried about my children having cancer than me getting cancer, and that's that horrific comment. You have to deal with a lot of psychological and social burden experienced by not only the parents but extended family members and providers. Seeing these kids go through this really takes a toll on us as well.

Interviewer: And so the treatment, if a limb removal is necessary or there's some sort of surgery necessary you have to do it multiple times because the children continue to grow?

Dr. Randall: That's a great question. It's a passionate issue in my life. The durability of the reconstruction is really important. You have to think about the growth of the child, but not only just the growth but the durability over decades of use down the road.

We use a lot of what we call endoprosthetics, which are sort of fancy knee and hip replacements that replace the bone as well as the joint in adults. But in children, we try, especially kids less than 10, to use what we call biological alternatives where we use their native anatomy in creative ways to enable them to have optimal function for decades.

Interviewer: Are there any, kind of, final thoughts you have if somebody is facing, themselves or a loved one, sarcoma diagnosis. It sounds like it's a very complicated cancer since there's how many different variations? Hundreds of them I think you said, could affect bone tissue, connective tissue, muscle tissue. What would be a final thought that you'd have for somebody as they're listening to this?

Dr. Randall: Well, if you're listening to this, don't panic for every lump and bump that you experience, see you're a practitioner, if you have concerns about it, especially if it's growing. If there is concern that it's a sarcoma make sure you go to a center with real experience. Here in Utah, but all around the country there are centers that are sarcoma centers, part of the National Conference of Cancer network and NCI designated cancer centers that have a team of providers with real experience, and that's where you want to be treated.

Interviewer: Because it's just, really, it's such a serious thing. It's really important that you have the best of the best and the experts really kind of assisting you through this process.

Dr. Randall: And the last thought also is, while it's an emotional emergency, it's not a medical one. And what I mean by that is if you're not comfortable with the information you're hearing, certainly get a second opinion. Make sure that you start the race, if you will, with your best foot forward. Don't just embark on the sake of the treatment because it's quick and convenient. But get all your ducks in a row because it can be a tumultuous ride.

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